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CROHN’S DISEASE IN CYSTIC FIBROSIS
H Chaun1, ECS Lam1, WCP Kwan1, PG Wilcox2
1Division of Gastroenterology, and 2Adult Cystic Fibrosis Clinic, University of British Columbia, Vancouver, British Columbia
Cystic fibrosis (CF) is a common lethal genetic disease affecting Caucasians, incidence 1:3200 with roughly 3500 CF patients in Canada. In a survey of 11,321 patients in 52 US CF clinics in 1992, there was a prevalence of Crohn’s disease (CD) of 1 in 404, 11 times higher than expected from a comparable aged non-CF control group. Since the Adult CF Clinic in Vancouver was established in 1981, 3 patients with CD have been encountered among over 300 patients seen.
Case 1. A 43 y.o. man, diagnosis of CF aged 4 months (deltaF508/N1303K mutations), manifested by rectal prolapse, lung infections and pancreatic insufficiency, presented with anal pain, large tender anal tags and perianal fistula at age 18. Rectal biopsy showed chronic active inflammation and non-caseating granulomata consistent with CD, He had chronic symptoms of left colonic CD and rectal stricture for years but since starting on azathioprine 4.5 years ago, his CD has been in sustained remission, with no worsening of his lung infections.
Case 2. A 47 y.o. woman, diagnosis of CF aged 18 (deltaF508/deltaF508) with characteristic lung infections, sinusitis and pancreatic insufficiency, had enterovesical and rectovaginal fistulae due to CD at age 30. Her CD has otherwise been manifested by prominent anal tags, anal fistula surgically excised (showing poorly formed granulomata) and patchy proctitis.
Case 3. A 27 y.o. woman, diagnosis of CF aged 23 (deltaF508/G551D) when she developed lingering respiratory infection. Abdominal cramping began at age 10 which became severe at age 23, with back pain, rectal bleeding and weight loss. Subsequent investigations showed fistulizing (rectovaginal) colonic CD. She has responded satisfactorily to 8-weekly infliximab infusions and maintenace azathioprine in the past 2 years, with no evident aggravation of her pulmonary status.
SUMMARY AND CONCLUSIONS: 1. With increased life expectancy of CF (median survival age now well over 40 years) and the recognized high incidence of CD in CF patients, CD should be considered in the investigation of CF patients with intractable intestinal symptoms. 2. As demonstrated in cases 1 and 3, the use of immunosuppressive and biologic agents for CD seem to be well tolerated without adverse effects on the pulmonary status of CF. Continued close monitoring in this regard is, however, clearly indicated.